Affiliation:
1. Sulaiman Al-Rajhi University: Sulaiman Al Rajhi Colleges
2. Ahmad Dahlan University: Universitas Ahmad Dahlan
Abstract
Abstract
Introduction:
Fahr’s syndrome (FS) is a rare neurodegenerative disorder characterized by bilateral and symmetrical calcification of basal ganglia and other brain structures. In contrast to Fahr’s disease, FS is distinguished by the association of secondary etiology. The diagnosis is confirmed by neuroimaging studies such as a head computer tomography (CT) scan or magnetic resonance imaging, which displays the calcification of basal ganglia and other structures in a bilateral and symmetrical pattern.
Case Report:
A 63-year-old female with a previous history of total thyroidectomy and ischemic stroke presented to our hospital with altered mentation and recurrent generalized tonic-clonic seizures. Laboratory evaluation revealed hypocalcemia, hypokalemia, and hypoparathyroidism. Imaging with head CT was consistent with FS, demonstrating bilateral, symmetrical, and extensive calcification of basal ganglia, corona radiata, and cerebellar hemispheres with ventricular enlargement.
Conclusion:
Our case represents a long-term consequence of untreated post-surgical hypoparathyroidism, which led to secondary FS. Long-term hypocalcemia and low calcium/phosphate ratio are associated with basal ganglia calcification in patients with hypoparathyroidism. Maintenance of eucalcemic and euphosphatemic state is essential as the preventive measure for metastatic calcification. There is currently no definitive treatment for FS, with adjunctive symptomatic treatments to reduce the clinical manifestations.
Publisher
Research Square Platform LLC
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